Journal of Clinical Oncology, Vol 11, 1985-1989, Copyright © 1993 by American Society of Clinical Oncology
Richter's syndrome: a report on 39 patients
LE Robertson, W Pugh, S O'Brien, H Kantarjian, C Hirsch-Ginsberg, A Cork, P McLaughlin, F Cabanillas and MJ Keating
Department of Hematology, University of Texas M.D. Anderson Cancer Center, Houston 77030.
PURPOSE: The incidence, clinical features, laboratory findings, and
treatment results of 39 patients with Richter's syndrome (RS) are reported.
PATIENTS AND METHODS: Thirty-nine of 1,374 patients with chronic
lymphocytic leukemia (CLL) developed RS. RESULTS: Features associated with
RS included systemic symptoms (59%), progressive lymphadenopathy (64%),
extranodal involvement (41%), elevation of lactate dehydrogenase (LDH;
82%), and a monoclonal gammopathy (44%). Analysis of the CLL karyotype
showed no specific chromosomal abnormality that conferred increased risk;
however, multiple abnormalities were common. Patients at all Rai stages and
in complete response (CR) were at risk, including three CR patients with no
residual disease at the level of detection by dual-parameter flow cytometry
or restriction analysis for immunoglobulin (Ig) gene rearrangements. The
incidence was not higher in patients who had received prior fludarabine or
chlorodeoxyadenosine. The median survival duration was only 5 months,
despite multiagent therapy. Patients who responded had prolonged survival
durations (P < .001). Three of eight patients who survived more than 1
year had a de novo presentation of both CLL and large-cell lymphoma (LCL).
Comparison of surface light- chain analysis from both low- and high-grade
components demonstrated isotypic light-chain expression in 12 of 15
patients. Ig heavy- and light-chain gene rearrangement analysis showed
identical rearrangement patterns in five of five patients. CONCLUSION: The
clinical, laboratory, and survival characteristics of our RS patients were
similar to those reported in earlier studies. Ig gene rearrangement and
light-chain isotype analysis support a common origin for CLL and LCL.
Despite progress in the treatment of CLL, the development of LCL remains a
serious complication and continued surveillance in all CLL patients is
warranted.
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